Liver & intrahepatic bile ducts
Hepatocellular carcinoma
Scirrhous type
Editorial Board Member: Raul S. Gonzalez, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Topic Completed: 8 April 2019
Minor changes: 9 October 2020
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PubMed Search: Scirrhous hepatocellular carcinoma[TIAB]
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Clinical features | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Fels Elliott DR, Gill RM. Scirrhous type. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumorscirrhousHCC.html. Accessed January 15th, 2021.
Definition / general
- Special type of hepatocellular carcinoma (HCC) designated by the WHO with abundant fibrous stroma separating nests and trabecula of tumor cells (Bosman: WHO Classification of Tumours of the Digestive System, 4th Edition, 2010)
Essential features
- Unencapsulated, often subcapsular location, may have satellite lesions
- Abundant fibrous stroma but lacks central scar or radiating fibrous bands
- No criteria defining the amount of fibrous stroma required for the diagnosis
Terminology
- Also called sclerosing variant of HCC
ICD coding
- ICD-10: C22.7 - Other specified carcinomas of liver
Epidemiology
- Incidence ranges from 0.2 - 4.6% of all HCC (J Gastroenterol Hepatol 2006;21:1470)
Clinical features
- No differences in patient demographics, presence of cirrhosis or serum alpha fetoprotein (AFP) levels in comparison with classic HCC (Surg Pathol Clin 2013;6:367)
- Association with hypercalcemia and hypophosphatemia has been described (Liver 1981;1:33)
Radiology description
- Radiographic appearance may be similar to cholangiocarcinoma
- Contrast enhanced CT scan shows peripheral ring enhancement in arterial phase and delayed central enhancement in venous phase (Eur J Radiol 2009;69:123)
Radiology images
Images hosted on other servers:
CT scans with scirrhous HCC
Prognostic factors
- Prognosis compared with classic HCC have found conflicting results (Surg Pathol Clin 2013;6:367)
- Prognostic factors for HCC: stage (TNM), single lesion versus multifocal, size, vascular invasion, portal vein thrombosis, severity of underlying liver disease (Liver Int 2009;29:502, J Surg Oncol 2018;117:644)
Case reports
- 60 year old man with cirrhosis and a 1.5 cm liver nodule (World J Gastroenterol 2009;15:2296)
- 67 year old woman with epigastric pain (Clin Gastroenterol Hepatol 2009;7:A28)
- 68 year old man with hereditary hemochromatosis (Semin Diagn Pathol 2017;34:126)
Treatment
- Surgical resection
- Radiofrequency ablation
- Transarterial chemoembolization (TACE)
- Transplantation (e.g. Milan criteria, modified by some institutions, Nat Rev Gastroenterol Hepatol 2017;14:203)
Gross description
- Unencapsulated, gray-white, firm, lobulated mass, often with serrated border
- Often subcapsular, may have satellite nodules
- More extensive fibrosis than fibrolamellar variant, no radiating fibrous bands, no central scar
- Gross appearance may be similar to intrahepatic cholangiocarcinoma
Gross images
Images hosted on other servers:
Scirrhous HCC
Microscopic (histologic) description
- Abundant fibrous stroma separating nests / trabecula of tumor cells
- No criteria defining the amount of fibrous stroma required for the diagnosis (25% and 50% have been used as cutoffs) (Pathol Int 2005;55:724, Eur J Radiol 2009;69:123, Hum Pathol 2018 Dec 28 [Epub ahead of print])
- Absence of fibrous capsule, no necrosis or hemorrhage, may contain intratumoral portal tracts and prominent tumor infiltrating lymphocytes (Pathol Int 2005;55:724, Hepatogastroenterology 2009;56:1086)
- Clear cell change, steatotic change and hyaline bodies may be present (J Gastroenterol Hepatol 2006;21:1470, Hum Pathol 2018 Dec 28 [Epub ahead of print])
- Less frequently poorly differentiated and more likely to have steatosis than nonscirrhous HCC (Hum Pathol 2018 Dec 28 [Epub ahead of print])
Microscopic (histologic) images
Contributed by Ryan M. Gill, M.D., Ph.D.
Unencapsulated
Fibrous stroma
Cytologic features
CK7
Glypican 3
HepPar1
Cytology description
- Cells are polygonal with distinct cell membranes, high N/C ratio, round nuclei, may have prominent nucleoli (similar to moderately differentiated HCC)
Positive stains
- Glypican 3+ and arginase1+ in 90% of cases (Mod Pathol 2013;26:782)
- CK7+, CK19+ and EpCAM+ in majority of cases (Histopathology 2005;47:382)
Negative stains
- HepPar1 and polyclonal CEA negative in > 50% (Surg Pathol Clin 2013;6:367)
- Mucin (e.g. mucicarmine)
Molecular / cytogenetics description
- Associated with TSC1 / TSC2 mutations (J Hepatol 2017;67:727)
Sample pathology report
- Liver, mass, core biopsy:
- Scirrhous hepatocellular carcinoma; see comment
- Comment: This tumor shows histologic features of scirrhous hepatocellular carcinoma with nests and trabecula of hepatoid cells within a dense fibrous stroma. The tumor cells show positive staining for glypican 3, arginase1 and CK7, as is typical for this variant.
Differential diagnosis
- Fibrolamellar variant:
- Intrahepatic cholangiocarcinoma:
- Metastatic carcinoma of pancreas:
- May form glands / tubules with cytoplasmic or intraluminal mucin
- Glypican 3-, arginase1-, albumin ISH-
- Epithelioid hemangioendothelioma:
- Myxoid stroma, "blister cells" with red blood cells in lumens or vacuoles
- CD34+, ERG+, CD31+, FLI1+, mucicarmine-, variable pankeratin, CAMTA1+, TFE3+ (subset) (Arch Pathol Lab Med 2009;133:967)
- Hepatocellular carcinoma with therapy induced fibrosis:
- May be difficult or impossible to distinguish from scirrhous HCC
- Check treatment history; look for other evidence of injury (e.g. necrosis, embolization beads)
Board review style question #1
Which of the following is true regarding this variant of hepatocellular carcinoma?
- Abundant eosinophilic cytoplasm
- Abundant fibrous stroma
- Central scar
- Lamellar fibrous bands
- Prominent nucleoli
Board review style answer #1
Board review style question #2
Which of the following is true regarding the scirrhous variant of hepatocellular carcinoma?
- Association with hypocalcemia has been described
- CK7 and CK19 are often negative
- It is associated with TSC1 / TSC2 mutations
- There is often necrosis or hemorrhage
Board review style answer #2
C. It is associated with TSC1 / TSC2 mutations
Reference: Scirrhous hepatocellular carcinoma
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Reference: Scirrhous hepatocellular carcinoma
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